Hearing Impairment in Patients with Behcet s Disease: Associations with Disease Activity and Clinical Manifestatioins

Introduction: Behcet s disease (BD) is an autoimmune multisystem disorder characterized by aphthous stomatitis, genital ulcerations and uveitis. Vasculitis is a key feature of the disease which may lead to functional disturbances in highly vascularized organs. BD has both cochlear and vestibular manifestations on the auditory system. The incidence of hearing loss in BD has been reported to be between 12 to 80%. Inner ear disturbances is the fourth to seventh most common clinical manifestation in BD. Audiologic assessment and management may be helpful for early diagnosis and treatment of patients with BD. The Aim of this work was to study the frequency, type, degree of hearing loss in BD patients and to assess cochlear function using otoacoustic emissions (OAE) and Electrocochleography (ECochG) aiming at early identification of sub clinical cochlear affection in BD patients. Correlation with disease activity, clinical manifestations and laboratory investigations were considered. Subjects and Methods: Thirty adult patients (23 males and 7 females) suffering from BD, diagnosed according to the International Study Group for Behcet s disease in1990 were included in the present study. The mean age of the patients was 33.47±7.22 years and mean disease duration was 8.77±6.57 years. Twenty age and sex matched healthy volunteers served as controls (15 males and 5 females) with a mean age of 33.9±8.9 years were also included in the present study. Full history taking and clinical examination of all patients as well as laboratory investigations were performed. Medications received by the patients were considered and patients receiving corticosteroids were not excluded. Disease activity was assessed using the Behcet s Disease Current Activity Form (BDCAF) score and according to the method of Aydintug and his colleagues (1995). Electrocochleography (ECochG) and otoacoustic emissions (TEOAE and DPOAE) were performed to all patients and controls. Results: Out of the 30 BD patients, 23 (76.7%) presented with normal hearing thresholds, 5 (16.7%) had mild hearing loss and 2 (6.7%) suffered from moderate hearing loss. Patients with normal hearing had shorter disease duration (5.30±3.09 years) than those presenting with hearing loss (10.29±5.05 years) (p=0.003). Cochlear pathology was centered around the mid and basal turns of the cochlea. These patients also exhibited a subclinical endolymphatic hydrops, which was not affected by disease activity but was influenced by duration of the disease. There was no relation between cochlear affection and any organ affection. Conclusion: BD has a definite affection on the auditory system. BD patients demonstrated a sub clinical vestibular hydrops. Cochlear pathology in BD patients was centered around the mid and basal turns of the cochlea. Effect of duration of the disease was mainly on the sub clinical vestibular hydrops rather than cochlear pathology. Screening patients with BD for the presence of cochlear pathology might be cost effective as the disease duration increases.